• Purkinje cells show impaired mitochondrial bioenergetics, with a deficiency in respiratory chain complexes I and III, leading to increased levels of protein carbonylation and mitochondrial reactive oxygen species production

• mice show reduced ATP synthesis in mitochondria from cerebellum, but not from entire brain or spinal cord, at 6 months with pyruvate, glutamate, and succinate/rotenone, which becomes more severe at 12 months of age